Collagen Vascular Disorders

This is a group of disorders including-Lupus Erythematosous, The Antiphospholipid Syndrome, Dermatomyositis, Scleroderma, Lichen myxoedematosus, Morphoea, Lichen sclerosis and Relapsing Polychondritis.

Some of these conditions are extremely rare and are not described here. Other conditions like lupus, morphea, and anti-phospholipid syndrome are seen fairly regularly in the Dermatology clinic and have been discussed here.

Lupus Erythematosus-describes a spectrum of conditions ranging from a localised photosensitive skin eruption to a potentially fatal multi-system disease. Many forms of lupus mainly affect just the skin, such as Discoid lupus erythematosus and Subacute lupus erythematosus( SLE).

SLE is an auto-immune condition where the body’s own immune system attacks itself. SLE often presents as facial rash, triggered by sunlight. Females are more affected then males and it affects all races. A skin biopsy (where a sample of skin is sent off to the pathologist and examined under the microscope) is always necessary to confirm the diagnosis. Your Doctor may also suggest some blood tests.

There is no cure for lupus. However, there are a lot of things that can be done to improve it. Avoiding sunlight is essential. Broad spectrum sunscreens, and protective UV clothing is necessary (Hats, long-sleeves). Apply sunscreen 15-30 minutes before any sun exposure and this sunscreen will have to be re-applied very frequently. Avoid mid-day sun exposure between the hours of 11-3 pm. Sometimes a patient will need to install a special filter in the windows of the car/office/home to block UVA which is not blocked by normal glass. Vitamin D supplementation will need to be taken. This can be bought over the counter from the pharmacy or the Health food shop. Pregnant women need to be very closely monitored as sometimes the baby may develop heart block and require a pacemaker. Patients who develop the multi-system disease will need to be under the care of a multi-discipliniary team at the hospital.

Antiphospholipd Syndrome – is an often not recognised condition. Recurrent miscarriages, venous and arterial thromboses at a young age (sometimes when the oestrogen contraceptive pill is started) will raise suspicion amongst Doctors. Livedo reticularis, Raynaud’s phenomenon, digital ischaemia or gangrene, and thrombophlebitis may be seen as the Dermatological presenation. A blood test will be suggested by your Doctor to confirm.

Dermatomyositis – this is a rare condition that causes inflammation of the skin and muscles, leading to a progressive proximal muscle weakness. There is a distinctive mauve/purple discoloration with oedema/swelling often in a light-exposed distribution.

Scleroderma-this is a rare fibrotic skin disease characterised by excessive deposition of collagen and other connective tissue components leading to dermal fibrosis. Patients often presents with Raynaud’s syndrome (where the skin of the fingertips go white with the cold) and the skin can be tethered and hardened. A patient may have trouble extending their fingers fully, may have difficulties properly opening the mouth to speak/eat etc.

Morphea – this is a sclerosis of the skin. The underlying cause is unknown. Usually this is localised to one area and consists of a single, oval or round plaque with a smooth, shiny surface. It can be red/purple initially but sometimes resolves by itself, leaving a brown discolouration behind. The breast and abdomen are common sites. The local variety may respond to superpotent topical or injectable steroids. Sometimes a plastic surgeon will be necessary especially for linear morphea (coup de sabre), which may affect the face.

Lichen Sclerosis is an autoimmune disorder that causes white shiny paper-like skin mainly in female patients and mainly in the genital region. This has already been discussed on a separate page.